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Systemic Diseases and the Skin
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Jana Kazandjieva, Razvigor Darlenski, Nikolai Tsankov
Polycythemia vera is an example of a chronic myeloproliferative disorder of myeloid cells that results in an increased red cell mass. The vascular complications in patients with polycythemia vera include a plethora and flushing of the face and palms, aquagenic pruritus associated with a hot shower in 40% of patients), erythromelalgia, livedo reticularis, acrocyanosis, and pyoderma gangrenosum.
High altitude residents
Published in Andrew M. Luks, Philip N. Ainslie, Justin S. Lawley, Robert C. Roach, Tatum S. Simonson, Ward, Milledge and West's High Altitude Medicine and Physiology, 2021
Andrew M. Luks, Philip N. Ainslie, Justin S. Lawley, Robert C. Roach, Tatum S. Simonson
Monge made extensive studies of the ability of permanent residents of the Andes to withstand the hypoxia and cold of the environment. Nevertheless, he is best known for his work on chronic mountain sickness, also known as Monge's disease (discussed in Chapter 24), which he set out in his book La Enfermedad de los Andes (Monge 1928). In this book, he describes the condition associated with severe polycythemia, cyanosis, and a variety of neuropsychological complaints including headache, dizziness, somnolence and fatigue. Initially, the condition was thought to be polycythemia vera, but was later shown to be a distinct malady.
Radioisotopes in Biology and Medicine
Published in Kedar N. Prasad, Handbook of RADIOBIOLOGY, 2020
Polycythemia vera is a disease of unknown etiology characterized by an increase in the circulating red cell mass, which is caused by overproduction of these elements by the bone marrow and certain extramedullary sites — such as the liver and spleen. There is often an associated increase in the white cells of the myeloid series and the platelets. At present, it is generally accepted that 32P is the most effective and most easily administered form of treatment for this disease, although some observers are concerned about a possible increase in the incidence of leukemia as a result of 32P therapy. 32P has been found to be useful in the treatment of chronic leukemia, but not in acute leukemia. Of course, the 32P therapy of the chronic leukemias must be accompanied by proper systematic care and judicious use of transfusions and other affective agents, such as localized X-ray therapy, nitrogen mustard, antifolic acid preparations, urathene, and 6-mercaptopurine. 198Au is used in carcinoma of the prostate. Gamma radiation from 60Co is widely used in the treatment of several types of malignant diseases.
Enalaprilat and losartan decrease erythroid precursors frequency in cells from patients with polycythemia vera
Published in Hematology, 2023
Angela Bozza, Martina Bernardi, Daniela Catanzaro, Katia Chieregato, Anna Merlo, Giuseppe Astori
Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by the overproduction of red blood cells, often in association with leukocytosis and thrombocytosis [1]. Current first-line therapies are primarily directed at lowering hematocrit levels in order to decrease the risk of thrombotic events or cardiovascular complications, and comprise treatments with cytoreductive drugs, interferon-α and phlebotomy. After the discovery of a mutation in the Janus kinase 2 (JAK2V617F) that constitutively activates the JAK-STAT pathway and consequently cell proliferation in hematopoietic cells from PV patients, JAK2 inhibitors have been developed and tested as second-line therapies [1,2]. Despite these approaches, there is still the need for a major comprehension of the mechanisms involved in PV erythrocytosis in order to develop more effective therapies targeting this process.
Polycythemia vera: aspects of its current diagnosis and initial treatment
Published in Expert Review of Hematology, 2023
Richard T Silver, Ghaith Abu-Zeinah
Although significant advances have been made in our understanding of the biology, pathogenesis, and treatment of polycythemia vera, important issues remain pertaining to its diagnosis and treatment. Although JAK2V617F has become a marker for PV, it does not distinguish it clinically from phenotypically similar patients with essential thrombocythemia or the cellular phase of myelofibrosis. Errors in diagnosis may result. This is abetted by the unavailability of radioactive chromium and iodine to determine red cell and plasma volume, respectively, in the United States and in parts of Europe. In these instances, the bone marrow findings, emphasized by the World Health Organization in 2016 and 2022 are important and are helpful in more than 90% of the cases [36]. Evidence suggests that marrow examination is performed infrequently at major hematologic centers, and far less often in community practice. This leads to significant errors in diagnosis and treatment, and ultimately in survival.
Frequency of JAK2V617F, MPL and CALR driver mutations and associated clinical characteristics in a Norwegian patient cohort with myeloproliferative neoplasms
Published in Scandinavian Journal of Clinical and Laboratory Investigation, 2023
Susanne Lilleskare, Marta Vorland, Anh Khoi Vo, Aasne K. Aarsand, Håkon Reikvam
We found that 81% of patients with polycythemia vera in our study had JAK2V617F (Figure 2). The consensus in international literature is that over 95% of polycythemia vera-patients have a mutation in JAK2, with JAK2 exon 12 accounting for approximately 3%, and the remaining share attributed to JAK2V617F [1]. JAK2 exon 12 analysis was not included in our study, however exon 12 variants would still likely account for only a few of the missing cases. In 2016, Almedal et al. published results from a study of JAK2V617F in a Norwegian cohort and found that 75% of patients with polycythemia vera had JAK2V617F. There is some overlap between the participants included in that study and in our recent study. A likely overdiagnosis of polycythemia vera was described, and it was assumed that not all would meet the current diagnostic criteria [15]. This causal explanation is also likely applicable to our population. Further analysis of the polycythemia vera cohort was therefore not performed. Yet the percentage is closer to international consensus, and this could indicate that diagnostic precision has improved in the later years.